Background: Myomatosis Erythrocytosis Syndrome (MES)  is a paraneoplastic condition that presents with the simultaneous occurrence of uterine fibroids and secondary erythrocytosis, typically due to ectopic erythropoietin (EPO) secretion. The pathophysiology may involve local hypoxic stimuli or genetic mutations within the fibroid tissue that stimulate EPO gene expression. However, some patients, paradoxically, do not exhibit elevated EPO levels. Three main criteria involved in the diagnosis of the above mentioned presentation include:

1. Elevated hemoglobin levels.

2. Presence of a leiomyoma

3. Normalization of Hemoglobin levels after surgery.

Case Summary: A 49-year-old postmenopausal female presented with progressive abdominal distention and a palpable mass that had gradually increased over three years, with a notable acceleration in the last six months. Her laboratory tests revealed elevated hemoglobin (19 g/dL) and hematocrit of 17% with a normal EPO concentration (18.6 mIU/mL). Radiologic studies identified a solid mass measuring 17.6 x 23.5 x 26 cm, initially suspected to be a leiomyosarcoma. She underwent staging laparotomy with DJ stenting. Histopathology later revealed the mass to be a benign leiomyoma. Post-surgical recovery was marked by normalization of erythrocytosis.

Conclusion: This case illustrates that MES can occur even with normal serum erythropoietin levels, possibly due to intermittent or localized production of erythropoietin. A high index of clinical suspicion is the key to identifying this syndrome. Surgical management remains the diagnostic and curative modality of choice.

Ishika Sahu is pursuing her MS in Obstetrics and Gynecology at Sri Ramachandra Medical College and Hospital, Chennai, after completing her MBBS from R.G.Kar Medical College, Kolkata. She has presented multiple papers at national conferences. Her areas of interest include high-risk obstetrics, gynaecologic oncology, and gynaecological laparoscopy.